¹Sara Mukhtar, ¹Hudaa Mehkri, ¹Emad Khater

¹Sheick Khalifa Medical City

Introduction: ANCA vasculitis is a small-vessel vasculitis resulting in inflammation of small- and medium-sized blood vessels. Each type of AAV is a rare condition. The incidence is estimated to be between 10 and 20 individuals per million. The prevalence is estimated to be between 200 and 400 individuals per million.

Method(s): A 59-year-old male, known to have dyslipidemia, treated hepatitis C infection and recurrent URTI. He presented in December 2022 with one month history of cough with hemoptysis. Left ear pain with left eye redness associated with headache and small submandibular swelling noted 1 day prior to admission. No history of fever, weight loss or skin rash. Patient attended many hospitals and received many antibiotic courses without improvement.

He is an ex-smoker. He is allergic to celecoxib and sulfa containing drugs. His regular home medications are Aspirin and Statin.

Result(s): In regard to overall patient management, he received IV Methylprednisolone over 3 days 500 mg, 1000 mg, 1000 mg, continued on oral prednisolone initially 60 mg with a tapering regimen. Rituximab 1g, 500 mg and 500 mg each one week apart. Thiopurine Methyltransferase Activity was sent and patient was started on Azathioprine initially 100 mg daily which was increased to 150 mg daily in a week time. As the patient was allergic to sulfa containing drugs, he was started on aerosolized pentamidine for PCP prophylaxis.

The question to add cyclophosphamide to his previous management as hybrid regimen was raised as his creatinine was trending up, however it started to platue and improve gradually so cyclophosphamide was not added. Patient clinical and biochemical parameters improved and his repeat CT chest showed improvement and resolution of lung nodules. He only developed steroid induced DM for which he was managed with insulin and followed up by endocrinology team as outpatient.

Above management was in line with the KDIGO Guidelines for management of AAV, where induction of remission is achieved by glucocorticoids with either cyclophosphamide or rituximab. The RAVE study, a multicentre randomised control non inferiority trial of rituximab as compared with cyclophosphamide for remission induction concluded that rituximab is not inferior to daily cyclophosphamide treatment for induction of remission in severe AAV and may be superior in relapsing disease.

Maintenance therapy with either rituximab or azathioprine and low dose glucocorticoids is recommended after induction of remission. Optimal duration of the maintenance treatment is not known, but should be between 18 months and 4 years.

Conclusion(s): AAV has variable clinical manifestations, early recognition and diagnosis to decrease the risk of life-threatening complications is important. From renal point of view, kidney biopsy is the gold standard for diagnosis, but it should not delay starting immunosuppressive treatment, especially in patients who are rapidly deteriorating. Management of AAV has evolved significantly in the past two decades, with substantial improvements in survival and quality of life. However, more studies are needed especially in regard to duration of maintenance therapy.